|The skin is the largest human organ and because it is multifunctional, is often a ‘window’ as to what may be going on inside the body.
Most chronic skin conditions are managed in primary care, with input from secondary care when necessary. However, certain cases necessitate urgent referral to dermatology secondary care, for prompt management.
These include life-threatening conditions, but also common dermatoses, such as eczema and psoriasis, when normal function and quality of life are affected. This article gives an overview of urgent dermatology where there is a mortality risk.
Blistering skin disease, drug eruptions, eczema, emergencies, erythroderma, melanoma, psoriasis, skin cancer, urgent referral
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Patients with suspected skin cancer are usually referred as two-week wait referrals. Individuals who are most at risk of developing melanoma include those with Fitzpatrick type 1 skin (always burns, never tans), a positive family history of melanoma, frequent episodes of sunburn (peeling/blistering) from sun exposure or sunbeds, and atypical mole syndrome including >100 moles.
A typical presentation is an ‘odd’ or ‘ugly duckling’ mole that has changed in appearance or has become itchy or painful, or is bleeding spontaneously.
A lesion which is changing, asymmetrical and irregularly pigmented raises suspicions of developing into melanoma. Other features that cause concern are having different colours within the lesion, if it extends beyond the border and being larger than 6mm. The ABCDE acronym is used to identify lesions at risk of malignant transformation (see box 1).
Melanomas and non-melanoma skin cell cancers (such as squamous skin cancers or rarer sarcomas, lymphomas and Merkel cell carcinomas) all need to be referred urgently to dermatology in secondary care for treatment and management.
|Box 1: Red flag signs for early referral2|
|A Asymmetrical lesion
B Irregular border
C Multiple colours/irregular pigmentation
D Large lesion >6mm in diameter
E Evolving/changing lesion
Erythroderma is used to describe widespread and generalised erythema of the skin, affecting more than 90% of the body surface area.1 It is a good example of skin failure, when normal function and quality of life are affected.
There may be an associated exfoliative component a few days after the onset of erythema. Patients have intensely red, hot skin, which may be pruritic. They may be systemically unwell, with symptoms of fever, shivering, tachycardia, hypotension or even shock.
There are many causes, which may not always be obvious initially. These include atopic eczema, psoriasis, seborrhoeic dermatitis, drug reactions and, very rarely, cutaneous T-cell lymphoma, affecting all ages. These patients need to be managed in hospital because they can rapidly become very unwell – they are unable to maintain homeostasis in temperature and are at risk of fluid and protein loss, clotting abnormalities, potential infection and multi-organ failure.
Patients with eczema have fragile skin, which renders them susceptible to bacterial and viral skin infections.
Eczema herpeticum is caused when eczema is infected with herpes simplex virus type 1. These patients may have a fever and their topical treatments are not effective in treating the eczema.
The typical lesions are erythematous, punched-out ulcers, which may be weeping, itching and painful. There may be a history of having a cold sore. In children especially, symptoms may include not eating, drinking or sleeping well.
These patients need to be referred urgently. Viral and bacterial swabs are essential before commencing treatment with aciclovir and antibiotics.
Acute generalised pustular psoriasis
Acute generalised pustular psoriasis is a rarer subtype of acute psoriasis that is unstable and requires early admission.
It can be life-threatening and compromises normal function, so is a dermatology emergency. It presents as numerous sterile white pustules on erythematous, inflamed skin.
Triggering factors include sudden withdrawal of oral corticosteroids or potent topical steroids. Drugs such as beta-blockers and lithium can precipitate this condition. Infections and even pregnancy can also be triggers.
Complications of this condition can affect multisystems. Topical potent steroids, calcipotriol and emollients are used, sometimes in combinations with immunosuppressive agents such as ciclosporin, if there are no contraindications.
TEN causes loss of the full-thickness epidermal layer of skin (Photograph: Professor Rino Cerio/Royal London Hospital)
Toxic epidermal necrolysis
The most serious erythroderma, which is often drug-related and can evolve rapidly, is toxic epidermal necrolysis (TEN). Infections such as mycoplasma and viral infections have been implicated in this condition, but drugs are the most common cause (see box 2).
|Box 2: Common instigators of drug eruptions2|
|Antibiotics – sulfonamides, beta lactams, penicillins, macrolides
|Anticonvulsants – carbamazepine, lamotrigine, phenytoin|
|NSAIDs – ibuprofen|
|Antiretrovirals – nevirapine|
TEN is a rare, life-threatening condition that causes the loss of the full-thickness epidermal layer of skin and multi-organ involvement.2 There is also mucosal surface involvement of the eyes, mouth, oesophageal and genital areas.
These patients may present with a prodrome of malaise, fever, feeling unwell, oral mucosal ulceration and coryzal symptoms. Subsequently, they develop tender erythematous macules, which develop into blisters and then the epidermal layer of the skin is shed, often in sheets, revealing denuded dermis. Some of these macules become necrotic.
The Nikolsky sign is positive, whereby slight lateral pressure on the skin results in easy separation of the epidermis from the dermis. The drug causing this reaction may have been taken up to one month before the onset of symptoms.
Patients suspected of having TEN must be referred and admitted to hospital immediately. They usually require high dependency unit/intensive therapy unit care and are managed by a multidisciplinary team. The suspected drug is withdrawn immediately and supportive and nutritional care is the mainstay of treatment initially.
There is some evidence that IV immunoglobulin is of benefit in the early stages. Other systemic drugs, such as ciclosporin, have also been used. The severity of illness score for TEN (SCORTEN) is used to predict the severity and risk of mortality of the disease (see table 1).3
|Table 1: SCORTEN scoring system3|
|Age >40 years
|Heart rate >120bpm||1|
|Initial body surface area skin shed >10%||1|
|Serum urea >10mmol/L
|Serum glucose >14mmol/L||1|
|Serum bicarbonate <20mmol/L
|Evidence of malignancy||1|
Stevens-Johnson syndrome (SJS) is similar to TEN, but more limited, and is part of a spectrum. The body surface area of epidermal detachment is usually less than 10%. There is more severe mucosal involvement.
The mouth and lips exhibit extensive painful ulcers. There is significant conjunctival injection and risk of corneal damage. The list of drugs implicated is the same as for TEN (see box 2). Both TEN and SJS affect all ages. In children, however, there is an increased association with infections such as herpes simplex virus, Epstein-Barr virus, cytomegalovirus, hepatitis and mycoplasma causing SJS.
Patients present with a similar prodrome of flu-like symptoms, with fever, sore throat, cough, sore red eyes and malaise. Thereafter, targetoid erythematous macules develop on the body. These are painful and may blister, spreading rapidly to the face and the rest of the body.
Management is the same as for TEN – immediate discontinuation of all suspected drugs. Supportive care and treatment of any underlying cause are also initiated.
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is another serious skin disorder, caused by adverse reactions to a drug which also affects multisystems.
The drug implicated may have been commenced two to eight weeks previously (see box 3).
Patients present with a high fever and extensive generalised rash. This may be morbilliform (measles-like) or targetoid, with associated facial oedema including ear swelling. There is also mucosal involvement. Patients are often very unwell and need urgent referral to hospital.
Blood tests may reveal an eosinophilia, deranged LFTs, haematological abnormalities and other organ involvement. Supportive treatment is required. Systemic steroids and immunomodulators may also be used.
|Box 3: Drugs that might cause DRESS syndrome2|
Blistering skin disease
Bullous pemphigoid is most common in men aged over 60 years. They present with pruritic skin initially, which can develop into a rash and subsequently tense blisters/bullae over their body. The fluid within these blisters may be clear or haemorrhagic.
Trauma or rupture of the bullae causes denuded skin, making the patient more susceptible to skin infections.
If a large body surface area is covered in blisters, it becomes more difficult to maintain homeostasis. Temperature, BP and fluid balance require close monitoring.
As for most blistering conditions, these patients need urgent confirmation of the diagnosis with a skin biopsy and other investigations. There is an increased risk of underlying malignancy, which must be investigated. Usually topical steroids are used in combination with oral steroids, steroid-sparing agents or a tetracycline in the first instance. Maintenance immunosuppressive therapy is required, often for years.
Pemphigus vulgaris usually occurs in younger individuals and can be more challenging to manage because lifelong therapy is often required.
Unlike bullous pemphigoid, it is difficult to see intact blisters, because these are flaccid and form higher up in the skin, usually within the epidermis. Patients are more likely to have additional involvement of the mucosal surfaces, requiring a multidisciplinary approach.
- Dr Beebee Meeajun is a specialty training registrar in dermatology and Professor Rino Cerio is consultant dermatologist and professor in dermatopathology at Bart’s Health NHS Trust, London
Competing interests: None declared
1. Mistry N, Gupta A, Alavi A et al. A review of the diagnosis and management of erythroderma (generalized red skin). Adv Skin Wound Care 2015; 28: 228-36.
2. Burns T, Breathnach S, Cox N et al (editors). Rook’s Textbook of Dermatology (eighth edition). Oxford, Wiley-Blackwell, 2010.
3. Bastuji-Garin S, Fouchard N, Bertocchi M et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol 2000; 115: 149-53.
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